Parkinson’s Disease and other related movement disorders – a video guide to diagnosis
Parkinson’s DiseaseMasterclass November 2017
Dr Frank Phelan
MidYorkshire Hospitals NHS Trust
Ideopathic Parkinson’s Disease
Core Motor Symptoms
Bradykinesia
Generalised slowness of movementUsually starts with decreased manual dexterity of the fingersIn the legs get dragging of feet, shorter shuffling steps or unsteadinessIn examining assess the speed, amplitude and rhythm of finger tapping, hand gripping, pronation-supination of hand movements and heel and toe tapping
PTO
Ideopathic Parkinson’s Disease
Core Motor Symptoms
Bradykinesia
As the disease progresses get Gait freezing and festination (an irresistible impulse to take much quicker and shorter steps and therefore to adopt unwillingly a running pace)
Ideopathic Parkinson’s Disease
Core Motor Symptoms
Rigidity
Increased resistance to passive movement
Often begins unilaterally (on same side as the tremor (if present))
Many patients develop cogwheel rigidity (a ratchety pattern of resistance and relaxation as the limb is moved through its full range of motion)
Can affect any part of the body – striatal hand (extension of the proximal and distal interphalangeal joints and flexion of the metacarpophalangeal joints), decreased arm swing and stooped posture
Ideopathic Parkinson’s Disease
Core Motor Symptoms
Tremor
Pill rolling rest tremor most noticeable when tremulous limb is supported by gravity and not engaged in purposeful activities
Can be present with action but generally more severe at rest
Some have a re-emergent tremor when the tremor re-emerges after several seconds and has a frequency similar to the rest tremor
PTO
Ideopathic Parkinson’s Disease
Core Motor Symptoms
TremorIn early PD the tremor often intermittent, starting unilaterally and spreads contralaterally after several years
Distracting the patient by asking to perform mental calculations or voluntary repetitive movements of the contralateral limb often accentuates the tremor
Can also involve the legs, lips, jaw and tongue but rarely the head (contrast this tremor with that of Essential Tremor and Dystonic Tremor)
Ideopathic Parkinson’s Disease
Core Motor Symptoms
Postural Instability
Usually does not appear until later in the course of PD. Tested with the ‘Pull –test’- patient with normal postural reflexes should be able to maintain balance and retropulse (step backwards) no more than one step. Patients with PD and postural instability are likely to fall or take multiple steps backwards
Patients who fall earlier most likely have another Parkinsonian syndrome such as Progressive Supranuclear Palsy or Multisystem Atrophy
Ideopathic Parkinson’s Disease
Core Motor Symptoms
Bradykinesia
Rigidity
Tremor
Postural Instability
Pre – Motor symptoms of PD
Ideopathic Parkinson’s Disease
Other Motor Features
CraniofacialHypommiaDecreased spontaneous eye blinking Speech Impairment
Hypokinetic dysarthriaHypophoniaPalilalia
DysphagiaSialorrhoea
Ideopathic Parkinson’s Disease
Other Motor Features
Visual
Hypometric saccades
Impaired vestibulocular reflex
Impaired upward gaze and convergence
Eye lid opening apraxia
Ideopathic Parkinson’s Disease
Other Motor Features
Musculoskeletal
MicrographiaDystoniaMyoclonusStooped PostureCamptocormiaPisa SyndromeKyphosis and scoliosis
Ideopathic Parkinson’s Disease
Non - Motor Symptoms
Cognitive Dysfunction and Dementia
• Approx 80% will develop dementia during the disease• Older age and severity of PD motor symptoms are associated with an increased
risk of developing dementia• The dementia is classically considered a subcortical dementia • Dementia usually occurs late in the course of PD (compare this with Dementia
with Lewy Bodies)
•
Ideopathic Parkinson’s Disease
Non - Motor SymptomsCognitive Dysfunction and Dementia
• Problems of Executive function (decision making and multi-tasking) are often the first signs of disturbed cognition
• Clinically, patients are diagnosed with PD dementia if their illness begins with PD and they develop dementia at least one year after the onset of Parkinsonsmotor symptoms
Ideopathic Parkinson’s Disease
Non - Motor Symptoms
Psychosis and Hallucinations
• Visual hallucinations are the most common psychotic symptom
• Can be attributable to PD itself or to the anti=Parkinsonian Treatment (more likely with Dopamine agonists)
• Delusions area also prominent and are usually paranoid in nature
Ideopathic Parkinson’s Disease
Non - Motor Symptoms
Mood Disorders
• Depression - very common and often predates the diagnosis
• Anxiety
• Apathy - less prevalent in those with higher levodopa equivalent dosage (mainly dopamine agonists)
Ideopathic Parkinson’s Disease
Non - Motor Symptoms
Sleep Disturbance
• Insomnia
• Daytime sleepiness with sudden onset of sleep attacks
• Restless legs syndrome
• REM Sleep Behaviour Disorder
Ideopathic Parkinson’s Disease
Non - Motor Symptoms
Sleep Disturbance
The most common causes for sleep awakenings in PD are nocturia, difficulty turning over in bed, cramps, vivid dreams or nightmares and pain. Tremor may also contribute as does painful dystonias in some
Fatigue
Ideopathic Parkinson’s Disease
Non - Motor Symptoms
Autonomic Dysfunction
Postural hypotension
Constipation
Dysphagia Also present in MSA but generally more
Diaphoresis severe than PD
Urinary difficulties
Sexual dysfunction
Ideopathic Parkinson’s Disease
Non - Motor Symptoms
Olfactory Dysfunction
Common and may precede motor symptoms or occur relatively early in the course of PD
May not be noticed by patients
Pain
Painful sensory symptoms common
- lancinating, burning, tingling, generalised or localised
Tends to correlate with motor fluctuations
Ideopathic Parkinson’s Disease
Non - Motor Symptoms
Pain
Dystonia which is often painful can occur in early PD or when levodopa wears off
Morning dystonia affecting the foot is a common ‘off’ response to abstinence from levodopa during sleep
Progressive Supranuclear Palsy
Main Features
Progressive supranuclear ophthalmoplegia
Gait disorder, postural instability and falls
Dysarthria
Dysphagia
Rigidity
Frontal Cognitive disturbance
Mean age of onset 65yrs which is older than PD and almost no cases < 40yrs
Progressive Supranuclear Palsy
Gait
Stiff and broad based
Tendency to have their knees and trunk extended (as opposed to flexed posture of PD)
Arms slightly abducted
Impulsivity (probably from frontal lobe involvement)
Tend to pivot quickly rather than turn en block
Usually fall backwards
Gait and balance problems may worsen with Levodopa treatment
Progressive Supranuclear Palsy
Oculomotor Findings
Supranuclear ophthalmoplegia may take as long as 10 years to develop
Get a slowing of vertical saccades followed by a limitation of saccadic range
Concomitant limitation of lateral gaze is often present
Pursuit movements of the eyes are slow, jerky, and hypometricwith unstable fixation
The ophthalmoparesis is initially overcome by the Oculocephalic (Doll’s eyes) manoeuvre
Progressive Supranuclear Palsy
Oculomotor Findings
Blepherospasm
Eye lid opening apraxia
The combination of rare blinking, facial dystonia, and gaze abnormalities leads to the development of a classical facial expression of perpetual surprise or astonishment
Progressive Supranuclear Palsy
Motor Involvement
Bradykinesia with marked micrographia
Axial rigidity more apparent especially neck and upper trunk
Retrocollis in some patients
One third have pyramidal signs with hyperreflexia and Babinski sign
Spastic dysarthria, dysphonia and dysphagia are profound in the middle to later stages
Stuttering and Palilalia
Progressive Supranuclear Palsy
Cognitive Abnormalities
Early and severe frontal (executive) deficits is a common finding
Pseudobulbar palsy is another characteristic feature
Hoarse groaning voice
REM sleep behaviour disorder is only infrequently seen in PSP and this combined with intact olfaction can help differentiate PSP from PD and MSA
Most often affects the hands and arms bilaterally but can be asymmetric
Can also affect the head and voice and uncommonly the face legs and trunk
Varies from a low amplitude, high frequency postural tremor of the hands to a much larger amplitude tremor that is attenuated by particular postures and actions
Becomes immediately apparent in the arms when they are held outstretched and typically increases at the very end of goal directed movements
Tremor of the legs unusual and PD more likely
Head tremor may be ‘yes-yes’ or horizontal ‘no-no’ and is usually associated with tremor of the hands or voice
By definition tremor should be the only neurological manifestation of ET
Can get cog-wheeling but without rigidity
Some patients develop enhanced physiological tremor due to anxiety
A positive family history (autosomal dominant) is supportive of the diagnosis
There may be a beneficial response to alcohol
DAT scan can reliably distinguish PD and other
Parkinsonian syndromes (MSA, PSP and CBD) from Essential Tremor
Main Features
Akinetic-rigid Parkinsonism
Autonomic Failure
Cerebellar ataxia
Pyramidal signs
Rapid progression regardless of dopaminergic treatment
Two phenotypes
MSA - P (Parkinsonism) and MSA- C (Cerebellar)
Multi- System Atrophy
MSA - P
Akinesia/bradykinesia
Rigidity
Postural instability and falls (usually within 3 years of motor onset)
Irregular, jerky postural and action tremor
Can get a rest tremor in up to one third of patients
MSA - P
Also can get:
Stimulus-sensitive cortical myoclonus
Hemiballism and chorea
Dystonia unrelated to Dopaminergic treatment
Orofacial dystonia or dyskinesia
PISA syndrome
Camptocormia
Speech – increased in pitch and quivering, strained element
Multi- System Atrophy
MSA - C
Gait ataxia
Limb ataxia
Ataxic dysarthria
Cerebellar dysfunction of eye movements
Cerebellar scanning dysarthria
Multi- System Atrophy
Sleep and breathing disorder
At least 1/3 get RBD1/3 develop nocturnal and diurnal laryngeal stridor
(and a risk for sudden death)
Also early features (and earlier and more severe than PD) get
Urinary frequency and urgency Incontinence Incomplete bladder emptying
Nearly all men develop early erectile dysfunction
Dysautonomia is a feature of both (Urinary dysfunction, Orthostatic hypotension)
Dysphagia common in both types
Multi- System Atrophy
Cognitive Function
Relatively well preserved compared with PD and other atypical Parkinsonian syndromes
Can develop emotional incontinence (pseudobulbar affect) – crying inappropriately without sadness or laughing inappropriately without mirth
Multi- System Atrophy
Have an association with Raynaud’s phenomenon
Usually there is a poor or un-sustained response to Levodopa
Levodopa induced unilateral facial dystonic spasms are particularly suggestive of MSA
Dementia with Lewy Bodies
Fluctuating cognition with pronounced
variation in attention and alertness
Recurrent visual hallucinations that are
typically well formed and detailed
REM sleep behaviour disorder which may
precede cognitive decline
One or more spontaneous cardinal
features of Parkinsonism (Bradykinesia, rigidity or
rest tremor)
Supportive Clinical Features
• Severe sensitivity to anti-psychotic agents
• Postural instability and repeated falls
• Syncope or other transient episodes of unresponsiveness
• Severe autonomic dysfunction
• Hypersomnia
• Hyposmia
• Delusions
• Hallucinations
• Apathy, anxiety and depression
Dementia with Lewy Bodies
Cognitive Dysfunction
• Often the presenting symptom
• Early impairment in attention and executive function (unlike AD when memory loss is the first feature)
• Early symptoms include driving difficult, misjudging distances, impaired job performance
• Early appearance of impaired figure copying (overlapping pentagons), clock drawing and seriel 7’s (or spelling WORLD backwards)
• Fluctuation in cognition and level of alertness may occur early in the course of DLB
Dementia with Lewy Bodies
Cognitive Dysfunction
• Fluctuation in cognition and level of alertness may occur early in the course of DLB
• There may be a brief decline in ability to perform an ADL and may be dramatic enough to raise the possibility of a stroke or seizure
• Patients can appear to ‘black out’ or lose consciousness, become confused or behave in a bizarre manner
• Can become excessively somnolent
Dementia with Lewy Bodies
Cognitive Dysfunction
• Visual hallucinations are an early sign in DLB and may precede Parkinsonism
• RBD commonly associated with DLB often early in the course of the disease. Can precede the diagnosis of DLB by 20 years
Main Features
Progressive asymmetric movement disorder
Symptoms initially affect one limb
Various combinations of:
akinesia, extreme rigidity, dystonia, focal monoclonus, ideomotor apraxia and alien limb phenomena
Cognitive Impairment
Parkinsonian features may present later
Cognitive features include executive dysfunction, aphasia, apraxia, behavioural change and visuospatial dysfunction with relatively preserved episodic memory
Main Features
Rigidity can be profound in the limb (and less so axially)
Dystonia involves abnormal posturing of the hand and foot (toe curling and foot inversion) that quickly become fixed
Along with apraxia the affected limb is rendered useless
Gait is variable and can be similar to PD or be a wide based ‘frontal lobe’ or freezing gait
Tremor less frequent than PD and more postural irregular and jerky
Dysarthria is an early feature
Main Features
Oculomotor Dysfunction
Abnormal eye movements in around 1/3 at presentation
Pursuit eye movements slow and saccadic
Vertical saccades usually normal (unlike PSP)
Cortical Dysfunction
Characteristic of CBD
Cognitive impairment and Behavioural changes
Limb apraxia and alien limb
Aphasia
Depression
End of teaching series
Thanks for reading!
CBD – Alien limb
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CBD1
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Lewy Body Dementia
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Lewy Body Dementia
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MSA1
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Multi system atrophy 2
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PSP 2
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PSP Facial appearance and Gait
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PSP Gait
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Tremor and Alcohol
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Supranuclear palsy - PSP
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Benign Essential Tremor and DBS
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Essential Tremor
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PSP 1
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Dystonia
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Executive Function
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REM sleep behaviour disorder
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PD – Rigidity, Bradykinesia and tremor
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PD Postural Instability
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Freezing of Gait
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Bradykinesia
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Dystonic tremor
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This Photo by Unknown Author is licensed under CC BY-NC-ND
Pisa Syndrome
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Eyelid opening apraxia
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Saccades
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PD Tremor
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Pre motor symptoms for PD
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PD Rigidity
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Hypommia
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Camptocormia
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Palilalia
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